Recombinant Human Ataxin 3 protein (ab86706)

Overview

Description

  • NatureRecombinant
  • SourceEscherichia coli
  • Amino Acid Sequence
    • SpeciesHuman
    • SequenceMESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLE LILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQQQQQQ QQQQGDLSGQ SSHPCERPAT SSGALGSDLG DAMSEEDMLQ AAVTMSLETV RNDLKTEGKK

Specifications

Our Abpromise guarantee covers the use of ab86706 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    SDS-PAGE

  • Purity> 90 % SDS-PAGE.
    ab86706 is purified using conventional chromatography techniques. Endotoxin Level: < 1.0 EU per 1 µg of protein (determined by LAL method).
  • FormLiquid
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped at 4°C. Upon delivery aliquot and store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

    Preservative: None
    Constituents: 10% Glycerol, 50mM Sodium chloride, 20mM Tris HCl, 2mM DTT, pH 7.5

General Info

  • Alternative names
    • AT3
    • Ataxin 3
    • ataxin 3 variant h
    • ataxin 3 variant m
    • ataxin 3 variant ref
    • Ataxin-3
    • ATX3
    • ATX3_HUMAN
    • ATXN3
    • EC 3.4.22.
    • JOS
    • Josephin
    • Machado Joseph disease
    • Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3)
    • Machado Joseph disease protein 1
    • Machado-Joseph disease protein 1
    • Machado-Joseph disease protein 1 homolog
    • MJD
    • MJD gene
    • MJD1
    • Olivopontocerebellar ataxia 3
    • OTTHUMP00000221583
    • OTTHUMP00000221585
    • OTTHUMP00000221586
    • OTTHUMP00000221587
    • OTTHUMP00000231995
    • OTTHUMP00000231997
    • Rsca3
    • SCA3
    • SCA3 gene
    • Spinocerebellar ataxia type 3 protein
    see all
  • FunctionInteracts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription. Acts as a histone-binding protein that regulates transcription. Acts as a deubiquitinating enzyme.
  • Tissue specificityUbiquitous.
  • Involvement in diseaseDefects in ATXN3 are the cause of spinocerebellar ataxia type 3 (SCA3) [MIM:109150]; also known as Machado-Joseph disease (MJD). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA3 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. The molecular defect in SCA3 is the a CAG repeat expansion in ATXN3 coding region. Longer expansions result in earlier onset and more severe clinical manifestations of the disease.
  • Sequence similaritiesContains 1 Josephin domain.
    Contains 3 UIM (ubiquitin-interacting motif) repeats.
  • Cellular localizationNucleus matrix. Predominantly nuclear, but not exclusively, inner nuclear matrix.
  • Information by UniProt

Recombinant Human Ataxin 3 protein images

  • 15% SDS-PAGE showing ab86706 at approximately 42.4kDa (3µg).

References for Recombinant Human Ataxin 3 protein (ab86706)

ab86706 has not yet been referenced specifically in any publications.

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